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Restrictive Cardiomyopathy

Topic Overview

What is restrictive cardiomyopathy?

Restrictive
cardiomyopathy is a serious problem that makes your heart muscle stiff. When
your heart muscle is stiff, it can't stretch to allow enough blood to enter its
lower chambers, the
ventricles. So blood that would normally enter the
heart backs up in your circulatory system.

Most of the time, this
leads to
heart failure. Heart failure doesn't mean that your
heart stops pumping. It means that your heart can't pump enough blood to meet
your body's needs.

What causes restrictive cardiomyopathy?

Often the
cause is never found. But we do know that there are a number of diseases or
problems that can lead to restrictive cardiomyopathy. These include:

Cardiac amyloidosis, a buildup of an
abnormal protein in the heart muscle.

Carcinoid syndrome, a rare disease that causes certain
chemicals to be released into the bloodstream. These chemicals can stiffen
heart muscle.

Löeffler's syndrome and endomyocardial fibrosis,
conditions that can cause scar tissue in the heart.

Genetic
factors. You can inherit diseases, including Gaucher disease and
Fabry's disease, that can lead to restrictive
cardiomyopathy. But these diseases can be treated to prevent restrictive
cardiomyopathy.

What are the symptoms?

You may not have any
symptoms at first. Or you may have mild symptoms, such as feeling very tired or
weak.

If your heart gets weaker, you will develop heart failure.
When this happens, you will feel other symptoms, including:

Shortness of breath, especially with
activity.

Tiredness.

Trouble breathing when you lie
down.

Swelling in your legs.

Heart failure that suddenly gets worse is an emergency. Get
medical help right away if:

You have severe trouble breathing.

You cough up pink, foamy mucus.

You have a new irregular or rapid heartbeat.

When you have heart failure, keeping track of your symptoms every day is important. Call your doctor if:

You have a sudden weight gain such as
3 lb (1.4 kg) or more in 2 to 3
days.

Your ability to exercise changes.

You have
any change in your symptoms.

How is restrictive cardiomyopathy diagnosed?

Your
doctor will ask questions about your symptoms and past health. He or she will
want to know about recent illnesses and about heart disease in your family.
Your doctor will listen to your heart and lungs and check your legs for fluid
buildup.

In some cases, a doctor may want to look at a small
sample of heart tissue, called a
biopsy, to make a definite diagnosis.

How is it treated?

Most of the time, treatment focuses on relieving symptoms, improving heart function, and helping you live longer. You may also have other treatment for the problem that is causing restrictive cardiomyopathy, such as medicines to get rid of too much iron in the heart muscle (hemochromatosis).

You will probably need to take
several medicines to treat heart failure caused by restrictive cardiomyopathy.
It's important to take your medicines exactly as your doctor tells you to
and to keep taking them. If you don't, your heart failure could get worse.

Your doctor may suggest a mechanical device to help your
heart pump blood or prevent life-threatening irregular heart rhythms. Such
devices include a
pacemaker,
an implantable cardioverter-defibrillator (ICD), or a combination pacemaker and ICD. If your
condition is very bad, a heart transplant may be an option.

Self-care is an important part of your treatment. Self-care includes the things you can do every day to feel better, stay healthy, and avoid the hospital.

Take your medicines as prescribed. This gives you the best chance of being helped by them. Some medicines for heart failure include:

Angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs). These make it easier for blood to flow.

Diuretics. These help remove excess fluid from the body.

Beta-blockers. These slow the heart rate and can help the heart fill with blood more completely.

Live a healthy lifestyle. This can help slow down heart failure. Limit salt, and don't smoke. Ask your doctor about how you can exercise safely. People who have heart failure from restrictive cardiomyopathy need to avoid doing too much, because their hearts can't increase blood flow during exercise.

Watch for signs that you're getting worse. Weighing yourself every day to watch for sudden weight gain is a good way to do this.

Find out what your triggers are, and learn to avoid them. Triggers are things that make your heart failure worse, often suddenly. A trigger may be eating too much salt, missing a dose of your medicine, or exercising too hard.

What can you expect with restrictive cardiomyopathy?

Most of the time, restrictive cardiomyopathy leads to heart failure.
Heart failure usually gets worse over time. But treatment can slow the disease
and help you feel better and live longer. If your doctor finds the cause of
your restrictive cardiomyopathy, then the cause will also be treated, if
possible.

Sudden cardiac death,
which means the heart suddenly stops working. This may be more likely to happen
to people who have serious rhythm problems (arrhythmias) in
one of the lower heart chambers (ventricles).

If your disease is getting worse, you may want
to think about making end-of-life decisions. It can be comforting to know that
you will get the type of care you want.

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Yancy CW, et al. (2013). 2013 ACCF/AHA Guideline for the management of heart failure: A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Journal of the American College of Cardiology, 62(16): e147–e239.

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